Table 1

 Demographic and clinical baseline characteristics of patients with PAH secondary to CTD at the beginning of the randomised studies

Bosentan-treated patients (n = 44)Placebo-treated patients (n = 22)
CI, cardiac index; CTD, connective tissue disease; mPAP, mean pulmonary arterial pressure; mRAP, mean right arterial pressure; PAH, pulmonary arterial hypertension; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; WHO, World Health Organization.
Data are taken from studies 351 and BREATHE-1. Results of the full dataset have been published in Channick et al8 and Rubin et al.9
Sex (men/women), n (%)6/38 (13.6/86.4)5/17 (22.7/77.3)
Age (years)
    Mean (SD)57.7 (12.6)49.7 (12.7)
    Range33.0–80.018.0–67.0
Race, n (%)
    Caucasian36 (81.8)19 (86.4)
    Black7 (15.9)1 (4.5)
    Other1 (2.3)2 (9.1)
Time from diagnosis (days)
    Mean (SD)791 (967)475 (716)
Aetiology of PAH, n (%)
    PAH/SSc37 (84.1)15 (68.2)
    SLE5 (11.4)3 (13.6)
    Overlap syndrome1 (2.3)3 (13.6)
    CTD (unclassified)1 (2.3)1 (4.5)
WHO functional class, n (%)
    III42 (95.5)21 (95.5)
    IV2 (4.5)1 (4.5)
Cardiac haemodynamics (mean (SD))
    CI (l/min/m2)2.4 (0.8)2.5 (0.8)
    PVR (dyn s/cm5)809 (451)722 (365)
    mPAP (mm Hg)47 (12)45.1 (10.6)
    mRAP (mm Hg)8.4 (5.1)8.0 (5.0)
    PCWP (mm Hg)8.9 (3.4)9.9 (5.1)
6-min walking distance (m)
    Mean (SD)312 (73)361 (67)